Primary Sjogren syndrome in adolescence: A case report and review

The finding of Sjogren syndrome (SS) in an adolescent female provided an opportunity to characterize the immunologic alterations of this disease and to monitor renal function before and after treatment with cyclophosphamide. Peripheral blood T4/T8 ratio was abnormally high at 2.55 with a normal number of T8 cells. Autologous mixed lymphocyte reaction and response to pokeweed mitogen stimulation were low. But, normal interleukin 2 production was consistent with the finding of normal numbers of T8 cells. Over time, renal function declined but returned to normal during cyclophosphamide therapy. After ten years, she continues to have a clinically stable course. SS is rare in children and adolescents. There is scant information on the nature and severity of this autoimmune disease in this age group. An increased awareness of this disorder may lead to the diagnosis of other afflicted children and a better understanding of the pathogenesis and clinical course of young people with SS.